March 31 Flashcards
What are the characteristics of Cushings syndrome?
Weight gain
Moon facies
Poor wound healing–caused by inhibition of collagen synthesis by glucocorticoids
Facial plethora
What disease is characterized by “starry sky” appearance seen on histo?
Burkitts lymphoma
Field of lymphocytes is interrupted by occasional phagocytic macrophage
What is the cause of X-linked hyper-IgM syndrome and how does it present?
Caused by CD40 ligand deficiency—unable to class switch so get increased IgM
Pt presents with recurrent pyogenic fungal infections–pneumocytitis is common
Normal or elevated IgM with major decrease in IgG, IgA, and IgE
Freely circulating IgM is capable of binding how many viral particles? What about an IgM bound to a B-cell?
Free-10–because it is a pentamer
Bound-2
What is the structure that forms the smooth part of both ventricles?
Bulbus cordis
Pathological grief includes….
Excessive, intense or prolonged grief
As well as grief that is delayed, inhibited or denied
B-hCG shares the identical alpha subunit of what other 3 hormones?
TSH
LH
FSH
Its the B subunit that makes these hormones different
What is Hartnup disease and how does it present?
AR disease that is a deficiency of neutral amino acid (tryptophan) transporters in proximal renal tubular cells and on enterocytes
Get neutral aminoaciduria and decreased absorption from the gut–leading to decreased tryptophan for conversion to niacin
Presents with pellagra-like symptoms–Diarrhea, Dementia and Dermatitis
What is decreased in both obstructive and restrictive lung disease?
Vital capacity
What are the functions of IL-1-6, 8, 10 and 12?
IL-1: Fever IL-2: Stimulates T cells IL-3: Stimulates Bone marrow IL-4: Stimulates IgE production IL-5: Stimulates IgA production IL-6: Stimulate aKute-phase protein production---C-reactive proteins "Hot T-Bone stEAK"
IL-8: Neutrophils are recruited to clear infections
“Clean up on aisle 8”
IL 10: AtTENuate the immune response
IL-12: Induces differentiation of T cells into Th1 cells and activates NK cells
What is the function of the sub thalamic nucleus, which is commonly damage with lacunar strokes causing hemiballisum?
Causes excitation of the internal segment of the globus pallidus which inhibits the thalamus leading to decreased motor cortex activation and movement
Therefore, when it is damage with stroke there is no longer inhibition of the thalamus which leads to the abnormal movements seen with hemiballisum
What are the treatment options for hodgkin lymphoma, which presents with a localized, single group of nodes and reed-sternberg cells?
Vincristine
Vinblastine
Bleomycin
Doxorubicin
What enzyme is deficient in Hunter syndrome, XR disorder that presents with developmental delay, gargoylism, airway obstruction, hepatosplenomegaly (all are features of Hurler syndrome which is more severe form of Hunters–Hurlers also presents with corneal clouding), and aggressive behavior WITHOUT corneal clouding?
Iduronate sulfates with accumulation of Heparan sulfate and dermatan sulfate
Hurler syndrome is caused by a-L-iduronidase deficiency and is AR disease
What are the most severe features of poliovirus?
Respiratory failure due to respiratory muscle paralysis
Paraplegia
Quadriplegia
What is the classic triad of Wiskott-Aldrich syndrome and what are the findings with this disease?
WATER–Wiskott-Aldrich
Thrombocytopenia
Eczema
Recurrent infections
Decreased to normal IgG and IgM
Increased IgE and IgA
Fewer and smaller platelets
XR and T cells unable to reorganize actin cytoskeleton
What is the cause of adult T-cell lymphoma that shows T-cells with multilobulated “cloverleaf” nuclei and presents with cutaneous lesions?
HTLV–provirus
What rotator cuff muscle is responsible for in aiding the deltoid in in abduction of the arm (especially first 15 degrees)?
Supraspinatus
SITS
- Supraspinatus
- Infraspinatus
- Teres minor
- Subscapularis
Methylmalonic acid is increased in ______ deficient due to its function to convert MMA to succinyl CoA.
Vit B12–cobalamin
What is Cinchonism and what is it seen with?
headache and tinnitus that can occur with class 1A anti-arrhythmics
What translocation is associated with Ewing’s sarcoma?
11;22
Blue anaplastic cells
What 2 test are specific for chronic granulomatous disease and what is defective in CGD?
Dihydrorhodamine–decreased green fluorescence
Nitroblue tetrazolium dye– get a negative test because there is a lack of reactive O2 species
Defect of NADPH oxidase–> decreased reactive O2 species and decreased respiratory burst in neutrophils
More susceptible to Catalase + species
What disease presents with the triad of cerebellar defects (ataxia), spider angiomas (telangiectasia), and IgA deficiency?
Ataxia-telangiectasia
What artery does the vertebral artery come off of?
Subclavian
Where is the classic location of a schwannoma?
Cerebellopontine angle
